المؤلفون: Okajima, Koichi, Kobayashi, Hiroshi, Suzuki, Hayata, Zhang, Liuzhe, Ishibashi, Yuki, Tsuda, Yusuke, Hinata, Munetoshi, Yasunaga, Yoichi, Ushiku, Tetsuo, Nakamoto, Yuji, Takeuchi, Yasuhiro, Tanaka, Sakae

المصدر: JOS Case Reports ; volume 3, issue 1, page 6-9 ; ISSN 2772-9648

الإتاحة: https://doi.org/10.1016/j.joscr.2023.09.011Test
https://api.elsevier.com/content/article/PII:S2772964823000473?httpAccept=text/xmlTest
https://api.elsevier.com/content/article/PII:S2772964823000473?httpAccept=text/plainTest

المؤلفون: Kobayashi, Hiroshi, Zhang, Liuzhe, Okajima, Koichi, Ishibashi, Yuki, Hirai, Toshihide, Tsuda, Yusuke, Ikegami, Masachika, Kage, Hidenori, Shinozaki‐Ushiku, Aya, Oda, Katsutoshi, Tanaka, Sakae

المصدر: Genes, Chromosomes and Cancer ; volume 62, issue 11, page 648-654 ; ISSN 1045-2257 1098-2264

الوصف: BRAF alterations, including V600E and non‐V600E mutations and fusions, in soft tissue sarcoma (STS) have been identified in a limited case series. Here, we aimed to evaluate the frequency of BRAF mutations and concurrent alterations in STS to understand their therapeutic action. In this retrospective analysis, we included data from 1964 patients with advanced STS who underwent comprehensive genomic profiling tests at hospitals in Japan between June 2019 and March 2023. The prevalence of BRAF and recurrent concurrent gene alterations were also investigated. BRAF mutations were detected in 24 (1.2%) of 1964 STS patients, with a median age of 47 (range 1–69) years. BRAF V600E was detected in 11 (0.6%) of the 1964 patients with STS, BRAF non‐V600E mutations in 9 (4.6%), and BRAF fusions were detected in 4 (0.2%). BRAF V600E was identified in 4 (0.2%) cases of malignant peripheral nerve sheath tumors. The most common concurrent alteration was CDKN2A (11 cases, 45.8%), and the frequency was equivalent to that of the BRAF V600E (5/11 cases, 45.5%) and non‐V600E (5/9 cases, 55.6%) groups. Recurrent concurrent alterations, such as TERT promoter mutations (7 cases, 29.2%), were detected at the same frequency in the V600E and non‐V600E groups. In contrast, TP53 alterations (4/9 cases, 44.4%) and mitogen‐activated protein kinase (MAPK)‐activating genes, including NF1 , GNAQ , and GNA11 (3/9 cases, 33.3%), were identified as relatively higher in the non‐V600E group than in the V600E group (each 1/11 case, 9.1%). We identified BRAF alterations at a rate of 1.2% in all patients with advanced STS. Among them, BRAF V600E and BRAF fusions account for 45.8% and 16.7%, respectively. Collectively, our findings support the clinical characteristics and therapeutic strategies for patients with BRAF ‐altered advanced STS.

الإتاحة: https://doi.org/10.1002/gcc.23182Test

المؤلفون: Takeyasu, Sho, Morita, Ken, Saito, Seitaro, Toho, Masanori, Oyama, Takashi, Obo, Takafumi, Taoka, Kazuki, Shimura, Arika, Maki, Hiroaki, Shibata, Eisuke, Watanabe, Yusuke, Suzuki, Fumio, Zhang, Liuzhe, Kobayashi, Hiroshi, Hinata, Munetoshi, Kurokawa, Mineo

المساهمون: Japan Society for the Promotion of Science, The University of Tokyo

المصدر: International Journal of Hematology ; volume 118, issue 6, page 745-750 ; ISSN 0925-5710 1865-3774

مصطلحات موضوعية: Hematology

الوصف: Myeloid sarcoma is a rare clinical entity that presents as an isolated proliferation of leukemic cells, concurrently with or at relapse of acute myeloid leukemia (AML), myelodysplastic syndromes/neoplasms (MDS), chronic myeloid leukemia (CML), and myeloproliferative neoplasm (MPN). Myeloid sarcoma disrupts the normal architecture of its surrounding tissues. When it forms in long bones, it can cause their pathological fracture. We recently experienced a rare case of MDS presenting with myeloid sarcoma in the femur that eventually resulted in its pathological fracture. Detailed chromosomal analysis of the bone marrow cells suggested emergence of myeloid sarcoma during the fast-paced progression of MDS just after acquiring trisomy 22. A comprehensive review of previous cases of myeloid sarcoma-associated pathological fracture indicated possible involvement of structural rearrangements of chromosomes 9 and 22. Management of myeloid sarcoma should continue to improve, and clinicians should note that myeloid sarcoma with specific chromosomal alterations needs extra medical attention to prevent pathological fracture.

الإتاحة: https://doi.org/10.1007/s12185-023-03656-1Test

المؤلفون: Tsuda, Yusuke, Yasunaga, Yoichi, Abe, Masanobu, Hoshi, Kazuto, Ito, Nobuaki, Kondo, Kenji, Okajima, Koichi, Zhang, Liuzhe, Kato, Hajime, Hidaka, Naoko, Tanaka, Sakae, Kobayashi, Hiroshi

المصدر: Oral and Maxillofacial Surgery Cases ; volume 9, issue 4, page 100335 ; ISSN 2214-5419

مصطلحات موضوعية: Otorhinolaryngology, Oral Surgery, Surgery

الإتاحة: https://doi.org/10.1016/j.omsc.2023.100335Test
https://api.elsevier.com/content/article/PII:S2214541923000445?httpAccept=text/xmlTest
https://api.elsevier.com/content/article/PII:S2214541923000445?httpAccept=text/plainTest

المؤلفون: Kobayashi, Hiroshi, Okajima, Koichi, Zhang, Liuzhe, Hirai, Toshihide, Ishibashi, Yuki, Tsuda, Yusuke, Ikegami, Masachika, Kawai, Akira, Tanaka, Sakae

المصدر: Japanese Journal of Clinical Oncology ; ISSN 1465-3621

الوصف: Background The clinical characteristics, outcomes, and prognostic factors of adult embryonal rhabdomyosarcomas (ERMS) and alveolar rhabdomyosarcomas (ARMS), particularly the differences among adolescents/young adults (AYA), adults, and older adults, remain unclear. We assessed the clinicopathological features and survival outcomes of adult patients with ERMS and ARMS in Japan and to compare these features among AYA, adult, and older adult patients. Methods We retrospectively analyzed data from the Bone and Soft Tissue Tumor Registry of Japan and enrolled patients aged ≥15 years with ERMS and ARMS. Disease-specific overall survival (DOS) was estimated using the Kaplan–Meier method, and a Cox regression model was used to identify prognostic factors. Results Among 184 patients with ERMS and ARMS (median age, 27 years; interquartile range, 18–49 years), a high rate of distant and regional nodal metastases was initially observed in 65 (35%) and 66 (36%) cases, respectively. Older age and distant metastasis at first presentation were statistically poor prognostic factors, and histological subtype and site of tumor origin were not associated with DOS. In patients with localized ERMS and ARMS, older age and nodal metastasis were poor prognostic factors; the 5-year DOS rates of patients with and without nodal metastasis were 23% and 72%, respectively. Conclusions Older patients with rhabdomyosarcoma had a dismal prognosis, and distant metastasis was a poor prognostic factor. The prognostic factors differed between adult and pediatric patients with rhabdomyosarcoma; biological analyses, such as genome analysis of adult rhabdomyosarcoma and clinical trials with pediatric oncologists, are needed to improve the prognosis of adult rhabdomyosarcoma.

الإتاحة: https://doi.org/10.1093/jjco/hyae053Test

المؤلفون: Okajima, Koichi, Kobayashi, Hiroshi, Ito, Nobuaki, Kato, Hajime, Ishibashi, Yuki, Zhang, Liuzhe, Tsuda, Yusuke, Tanaka, Sakae

المصدر: Japanese Journal of Clinical Oncology ; volume 54, issue 4, page 463-470 ; ISSN 1465-3621

مصطلحات موضوعية: Cancer Research, Radiology, Nuclear Medicine and imaging, Oncology, General Medicine

الوصف: Background extended curettage is generally used to treat infiltrative bone tumours. However, the extent of the curettage performed in previous studies remains unclear. This study aimed to investigate the efficacy of extended curettage for bone tumour-induced osteomalacia. Methods we included 12 patients with tumour-induced osteomalacia who underwent extended curettage at our hospital between 2000 and 2022. Extended curettage was applied in cases where tumour resection could cause functional impairment or necessitate complex reconstruction. We investigated patients’ clinical and oncological outcomes. Results patients had a mean age of 55 (24–81) years, and the median follow-up duration after surgery was 3.9 (1.0–14.0) years. The causative tumours were located in the pelvis and lumbar spine. Imaging revealed the tumours to be of the sclerotic, intertrabecular, lytic and mixed types. Intraoperative 3D fluoroscopy was used in 10 patients. Extended curettage with high-speed burring and adjuvant therapy with cauterization using an electric scalpel and ethanol resulted in a remission rate of 83%; no recurrence or metastasis was observed in cases of early postoperative biochemical remission. In cases where the causative tumour was at the lumbar spine and ischium close to the acetabulum, no postoperative biochemical remission was observed, and conservative treatment was continued. Except for one patient with a tumour in the lumbar spine, all patients could walk without a cane. Conclusions extended curettage for bone tumour-induced osteomalacia is oncologically and functionally favourable, especially in cases where resection of the causative tumour could cause functional impairment or necessitate complex reconstruction.

الإتاحة: https://doi.org/10.1093/jjco/hyad180Test
https://academic.oup.com/jjco/article-pdf/54/4/463/57174486/hyad180.pdfTest

المؤلفون: Kobayashi, Hiroshi, Okajima, Koichi, Zhang, Liuzhe, Hirai, Toshihide, Ishibashi, Yuki, Tsuda, Yusuke, Ikegami, Masachika, Kawai, Akira, Tanaka, Sakae

المصدر: Japanese Journal of Clinical Oncology ; volume 54, issue 4, page 471-478 ; ISSN 1465-3621

مصطلحات موضوعية: Cancer Research, Radiology, Nuclear Medicine and imaging, Oncology, General Medicine

الوصف: Background Pleomorphic rhabdomyosarcoma is a rare sarcoma in adults. The clinical characteristics, outcomes and prognostic factors associated with pleomorphic rhabdomyosarcoma remain unclear. Methods We retrospectively analyzed data from the Bone and Soft Tissue Tumor Registry of Japan, and enrolled patients with pleomorphic rhabdomyosarcoma. Disease-specific overall survival, local recurrence-free survival and distant metastasis-free survival were estimated using the Kaplan–Meier method; Cox regression model was used to identify prognostic factors. Results In total, 182 patients with pleomorphic rhabdomyosarcoma were included. Median age was 63 (range 20–95) years. The lower extremity (48%) was the most frequent tumor origin site, while head and neck were rare (4%). A total of 43 patients (24%) had distant or regional nodal metastases at first presentation. In all cases, the 2-year and 5-year survival rates were 66.3% and 54.1%, respectively. Distant metastasis was a significant poor prognostic factor (Hazard ratio 6.65; 95% confidence intervals, 3.00–14.75, P < 0.0001), with median survival of such patients being 9.4 (95% confidence intervals: 5.3–12.2) months. In 134 localized cases, the 2-year and 5-year survival rates were 91.5% and 68.3%, respectively. Large tumor size and older age were associated with poorer prognosis. Through data from localized and locally curative cases extracted and adjusted by propensity score matching, we found that perioperative chemotherapy did not improve disease-specific overall survival, distant metastasis-free survival or local recurrence-free survival. Conclusions Clinical characteristics and outcomes of pleomorphic rhabdomyosarcoma are similar to those of other high-grade soft tissue sarcomas. Pleomorphic rhabdomyosarcoma may be less chemosensitive, and a strategy other than the standard cytotoxic chemotherapy is required to improve its prognosis.

الإتاحة: https://doi.org/10.1093/jjco/hyad188Test
https://academic.oup.com/jjco/article-pdf/54/4/471/57174444/hyad188.pdfTest

المؤلفون: Kobayashi, Hiroshi, Okuma, Tomotake, Okajima, Koichi, Ishibashi, Yuki, Zhang, Liuzhe, Hirai, Toshihide, Ohki, Takahiro, Ikegami, Masachika, Sawada, Ryoko, Shinoda, Yusuke, Akiyama, Toru, Goto, Takahiro, Tanaka, Sakae

المصدر: Journal of Orthopaedic Science ; volume 27, issue 1, page 222-228 ; ISSN 0949-2658

مصطلحات موضوعية: Orthopedics and Sports Medicine, Surgery

الإتاحة: https://doi.org/10.1016/j.jos.2020.11.006Test
https://api.elsevier.com/content/article/PII:S0949265820303468?httpAccept=text/xmlTest
https://api.elsevier.com/content/article/PII:S0949265820303468?httpAccept=text/plainTest

المؤلفون: Kobayashi, Hiroshi, Zhang, Liuzhe, Okajima, Koichi, Ishibashi, Yuki, Hirai, Toshihide, Tsuda, Yusuke, Ikegami, Masachika, Kage, Hidenori, Shinozaki‐Ushiku, Aya, Oda, Katsutoshi, Tanaka, Sakae

المصدر: Genes, Chromosomes & Cancer; Nov2023, Vol. 62 Issue 11, p648-654, 7p

المؤلفون: Zhang, Liuzhe, Akiyama, Toru, Fukushima, Takashi, Iwata, Shintaro, Tsuda, Yusuke, Takeshita, Katsushi, Kawai, Akira, Tanaka, Sakae, Kobayashi, Hiroshi

المصدر: Japanese Journal of Clinical Oncology ; volume 51, issue 6, page 918-926 ; ISSN 1465-3621

مصطلحات موضوعية: Cancer Research, Radiology, Nuclear Medicine and imaging, Oncology, General Medicine

الوصف: Background Approximately 10% of the patients with soft tissue sarcoma show metastasis at initial diagnosis, and hence, poorer prognosis. However, the prognostic factors and whether definitive surgery for the primary lesion improves overall survival, especially when complete resection of metastasis is difficult, remain unclear. Methods This retrospective analysis was based on the Bone and Soft Tissue Tumor Registry in Japan. Patients with soft tissue sarcoma having metastasis at diagnosis were enrolled, excluding those with Ewing’s sarcoma, rhabdomyosarcoma and several other sarcomas with unique behavior and treatment strategies. Overall survival was estimated using the Kaplan–Meier method and compared among the common histologic subtypes. Multivariate analysis with the Cox regression model was used to identify the prognostic factors. Results In total, 1184 patients were included, with a median follow-up duration of 10 months (range: 1–83). The median overall survival was 21 months (95% confidence interval: 18.2–23.8). The multivariate analyses indicated that tumor size, grade and histologic subtypes significantly correlated with overall survival. Moreover, surgery for the primary lesion, in addition to surgery for metastases and chemotherapy, showed significant association with better survival. Conclusions The prognostic factors in patients with metastatic soft tissue sarcoma at diagnosis are generally similar to those in patients with localized disease. The overall survival in patients differed significantly according to histologic subtype. Surgical resection of primary lesions, especially those with a wide margin, may be an independent prognostic factor. Further studies are needed identify which subgroup of patients would benefit the most from primary lesion surgery.

الإتاحة: https://doi.org/10.1093/jjco/hyab033Test
http://academic.oup.com/jjco/article-pdf/51/6/918/38360235/hyab033.pdfTest